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From the Department of Neurology (Drs. Boeve, Silber, Benarroch, Petersen, Ahlskog, Matsumoto, and Knopman, A.M. Schmeichel), Sleep Disorders Center (Drs. Boeve and Silber), and Departments of Laboratory Medicine and Pathology (Dr. Parisi) and Psychology and Psychiatry (Dr. Smith), Mayo Clinic, Rochester, Mayo Alzheimer’s Disease Research Center (Drs. Boeve, Parisi, Dickson, Ferman, Smith, Petersen, and Knopman), Mayo Foundation, and Minnesota Regional Sleep Disorders Center (Drs. Schenck and Mahowald), University of Minnesota, Minneapolis, MN; and Neuropathology Laboratory (Dr. Dickson) and Department of Psychology and Psychiatry (Dr. Ferman), Mayo Clinic, Jacksonville, FL.
Address correspondence and reprint requests to Dr. B.F. Boeve, Mayo Clinic, 200 First Street SW, Rochester, MN 55905; e-mail: bboeve{at}mayo.edu
Objective: To determine if synucleinopathy pathology is related to REM sleep behavior disorder (RBD) plus dementia or parkinsonism.
Methods: The clinical and neuropathologic findings were analyzed on all autopsied cases evaluated at Mayo Clinic Rochester from January 1990 to April 2002 who were diagnosed with RBD and a neurodegenerative disorder. Ubiquitin and/or 
-synuclein immunocytochemistry was used in all cases. The clinical and neuropathologic diagnoses were based on published criteria.
Results: Fifteen cases were identified (14 men). All had clear histories of dream enactment behavior, and 10 had RBD confirmed by polysomnography. RBD preceded dementia or parkinsonism in 10 (66.7%) patients by a median of 10 (range 2 to 29) years. The clinical diagnoses included dementia with Lewy bodies (DLB) (n = 6); multiple-system atrophy (MSA) (n = 2); combined DLB, AD, and vascular dementia (n = 1); dementia (n = 1); dementia with parkinsonism (n = 1); PD (n = 1); PD with dementia (n = 1); dementia/parkinsonism/motor neuron disease (n = 1); and AD/Binswanger’s disease (n = 1). The neuropathologic diagnoses were Lewy body disease (LBD) in 12 (neocortical in 11 and limbic in 1) and MSA in 3. Three also had argyrophilic grain pathology. In the LBD cases, concomitant AD pathology was present in six (one also with Binswanger’s pathology, and one also with multiple subcortical infarcts).
Conclusion: In the setting of degenerative dementia or parkinsonism, RBD often reflects an underlying synucleinopathy.
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